Autoimmune pulmonary aveolar aroteinosis (aPAP) is a rare lung disease characterized by the build-up of grainy material in the alveoli (air sacs) of the lungs. This grainy material is composed mainly of phospholipid (a fat-like substance) and protein. Phospholipid and protein are the key components of lung surfactant, an important substance that coats the alveoli to prevent lung collapse and which promotes oxygen absorption by the lungs. We will use the first model for aPAP to examine the underlying immune mechanisms that cause aPAP. Insights gained through our research could translate into treatment for patients.
Update: In patients with aPAP, mechanisms that regulate the production and activation of immune cells called Csf2 cytokine-specific B cells are broken, leading to the production of disease-causing Csf2-specific antibodies. We have detected key differences in the type of Csf2-specific antibodies with aPAP. We have also identified key molecules that are required for disease that could serve as candidate therapeutic targets for aPAP patients.